PB1834: ACQUIRED FACTOR VII DEFICIENCY ASSOCIATED WITH ACUTE MYELOID LEUKEMIA
Authors: Haifa Hafsa, Zaineb Mlayah, Alaa Ghorbel, Bizid Inaam, Wiem Boufrikha, Sarra Boukhriss, Adnene Laatiri
Journal: HemaSphere (2023)

Topic: 3. Acute myeloid leukemia - Biology & Translational Research Background: While acquired factor VII (FVII) deficiency has been previously reported in at least 31 cases, only 4 prior reported cases of acquired FVII deficiency associated with acute myeloid leukemia (AML) have been described in the literature. We discuss through a new case of acquired FVII deficiency, the characteristics of this disease when it is associated with AML. Aims: Given the rarety of acquired FVII deficiency, especially associated with AML, we report this case in order to enrich bibliography as well as to better define the clinical, biological characteristics and to suggest a therapeutic approach. Methods: We encountered only one case of acquired FVII deficiency associated with AML in our department that we are going to interpret. Results: A 38-year-old Arab male with no significant personal or familial history involving hematological pathologies, or bleeding history, was admitted to our department with a one-week history of anemic syndrome. Physical examination was significant for pale conjunctivae, mucosal pallor and gingival hyperplasia. Initial laboratory tests revealed severe pancytopenia and circulating blasts suggestive of underlying leukemia. Initial coagulation panel on admission was significant for slightly low Prothrombin Ratio (PT) at 56 %, while fibrinogen levels and partial thromboplastin time were within normal limits. The new-onset and severe pancytopenia, circulating blasts on peripheral smear on presentation prompted us to pursue with bone marrow aspirate witch confirmed AML with cellularity of 80 % myeloid blasts, the phenotype of the neoplastic cells showed strong expression of CD13, CD33, CD14 and CD64 on flow cytometry. Cytogenetics revealed normal karyotype. The patient subsequently was treated with Idarubicine (12 mg/m2/day) for 3 days and Aracytine (200 mg/m2/day) for 7 days. Initially, a vitamin K deficiency was considered and the patient was supplemented with vitamin K injections. However, in the setting of the isolated and persistent PT prolongation, Prothrombin complex coagulation factors dosing showed a decrease only in FVII. During his hospital stay, the patient received multiple fresh frozen plasma for temporary mucosal bleeding syndrome without any impact on PT neither on FVII activity. Literature mentioned treating aFVII deficiency with steroids, so the patient received 10mg dexamethasone daily for only 5 days. In result, PT has increased from 56% to 67% and FVII level went from 30% to 46% without reaching normal levels. Steroids were stopped due to high blood pressure. One month after completion of induction, we have noticed spontaneous return to normal range of the PT, as well for FVII activity level (62%). All of the above was concomitant to the disappearance of circulating blasts on peripheral smear. The evaluation of the chemotherapy response showed cytological remission (table 1). Summary/Conclusion: This case underscores the importance of a careful evaluation of a prolonged PT in patients presenting with AML. Steroids have shown promising results that we can’t assess. The FVII deficiency, which spontaneously cleared after induction chemotherapy and with the disappearance of blasts, must motivate to discuss a possible link between factor VII deficiency and myeloid blasts. It would be of interest for future studies to prospectively evaluate coagulation and to specifically investigate FVII levels in AML patients. We should look deeper into mecanisms and possible cytogenetic abnormalities responsible for this association.Keywords: Complete remission, Coagulation factors, Acute myeloid leukemia, Corticosteroids